Atypical chronic lymphocytic leukemia (CLL) is still defined according to morphological criteria. However, deviance from the typical surface immunological profile suggests an atypical immunological-based CLL. A large cohort of patients with CLL was retrospectively evaluated aiming at assessing morphological (FAB criteria), immunophenotypical (two or more discordances from the typical profile), and clinical–biological features of atypical CLL. Compared to typical cases, morphologically atypical CLL showed a greater percentage of unmutated IgVH and CD38 positivity, and a higher expression of CD20. Immunophenotypically atypical CLL was characterized by more advanced clinical stages, higher expression of CD20, higher rate of FMC7, CD79b and CD49d positivity, and by an intermediate–high expression of membrane surface immunoglobulin, compared to typical cases. When patients were categorized based on immunophenotypic and morphologic concordance or discordance, no difference emerged. Finally, morphological features better discriminated patients’ prognosis in terms of time-to-first treatment, while concordant atypical cases showed overall a worse prognosis. Discordant cases by immunophenotype and/or morphology did not identify specific prognostic groups. Whether—in the era of molecular markers used as prognostic indicators—it does make sense to focus on morphology and immunophenotype features in CLL is still matter of debate needing further research.
非典型慢性淋巴细胞白血病（CLL）目前仍依据形态学标准进行定义。然而，与典型表面免疫学特征的偏离提示存在基于免疫学特征的非典型CLL。本研究回顾性评估了大规模CLL患者队列，旨在分析非典型CLL的形态学（FAB标准）、免疫表型（与典型特征存在两项及以上不一致）及临床生物学特征。与典型病例相比，形态学非典型CLL表现出更高比例的未突变IgVH和CD38阳性率，以及更强的CD20表达。免疫表型非典型CLL的特征在于：相较于典型病例，其临床分期更晚、CD20表达更高、FMC7、CD79b及CD49d阳性率更高，且膜表面免疫球蛋白呈中高强度表达。当根据免疫表型与形态学特征的一致性或不一致性对患者进行分类时，未发现显著差异。最终，形态学特征在首次治疗时间方面能更好区分患者预后，而免疫表型与形态学一致的非典型病例总体预后更差。免疫表型和/或形态学不一致的病例并未形成特定的预后分组。在分子标志物作为预后指标的时代，是否仍有必要聚焦于CLL的形态学和免疫表型特征，仍是需要进一步研究的争议性问题。
肿瘤(癌症)患者之家